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Wernicke- Korsakoff syndrome AM Report April 18, 2003 Nam D. Vo, MD
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Page 1: Wernicke Korsakoff Syndrome

Wernicke- Korsakoffsyndrome

AM ReportApril 18, 2003

Nam D. Vo, MD

Page 2: Wernicke Korsakoff Syndrome

DR. CARL WERNICKE(1848-1904)

Wernicke was born inTarnovitz, Poland. Interested in psychiatry, traditionally he studied anatomy initially and neuropathology later. He published a small volume on aphasia which vaulted him into international fame. In it was precise pathoanatomic analysis paralleling the clinical picture. He is best known for his work on sensory aphasia and poliomyelitis hemorrhagiasuperior.

Page 3: Wernicke Korsakoff Syndrome

DR. SERGEI KORSAKOV (1853-1900)

Korsakov was born in Russia.. Although he studied alcoholicpolyneuritis with mental symptoms, his "Korsakov’s psychosis" was the term used only when the mental disorder was accompanied by neuritis symptoms. Known as a humanitarian, he improved conditions in mental institutions. He was the first great psychiatrist in Russia. He is considered a "moral genius.”

Page 4: Wernicke Korsakoff Syndrome

Background

In 1881, Wernicke first described an illness in 3 pts. that consisted of triad:– paralysis of eye movements,– ataxia,– mental confusion

The patients:– 2 males with alcoholism – 1 female with persistent vomiting following sulfuric acid ingestion– All progressed to coma and death.– Wernicke detected punctate hemorrhages affecting the gray

matter around the third and fourth ventricles and aqueduct ofSylvius. He felt these to be inflammatory and therefore named the disease polioencephalitis hemorrhagica superioris.

Page 5: Wernicke Korsakoff Syndrome

Background

1887-1891: Korsakoff described the disturbance of memory in the course of long-term alcoholism in a series of articles. He called this syndrome psychosis polyneuritica, believing that these typical memory deficits, in conjunction with polyneuropathy, represented different facets of the same disease. The term Wernicke encephalopathy is used to describe the symptom complex of ophthalmoplegia, ataxia, and an acute confusional state. If persistent learning and memory deficits are present, the symptomcomplex is termed Wernicke-Korsakoff syndrome.

Page 6: Wernicke Korsakoff Syndrome

Pathophysiology

A deficiency of thiamine is responsible for the symptom complex manifested in Wernicke-Korsakoff syndrome, and any condition resulting in a poor nutritional state places patients at risk. – Heavy, long-term alcohol use is the most common association

with Wernicke-Korsakoff syndrome. Alcohol interferes with active gastrointestinal transport, and chronic liver disease leads to decreased activation of thiamine pyrophosphate from thiamine, aswell as a decreased capacity of the liver to store thiamine.

Thiamine is converted to thiamine pyrophosphate, which serves as a cofactor for several enzymes that function in glucose utilization. These enzymes include transketolase,pyruvate dehydrogenase, and alpha ketoglutarate.

Page 7: Wernicke Korsakoff Syndrome

Pathophysiology

Thiamine deficiency results in a diffuse decrease in cerebral glucose utilization. However, symptoms are attributed to focal areas of damage.– Ocular motor signs: lesions in the brainstem affecting the

abducens nuclei and eye movement centers in the pons and midbrain.

– Ataxia: damage to the cerebellum, particularly the superiorvermis. The loss of neurons leads to persistent ataxia of gait and stance. In addition to cerebellar dysfunction, the vestibularapparatus also is affected.

– Memory impairment: damage in the diencephalon, including the medial thalamus, and connections with the medial temporal lobes and amygdala. The slow and incomplete recovery of memory deficits suggests that amnesia is related to irreversible structural damage.

Page 8: Wernicke Korsakoff Syndrome

Pathophysiology

The mechanism by which thiamine deficiency leads to damage in these specific areas is not fully understood.Proposed mechanisms:

– altered cerebral energy metabolism resulting from decreases intransketolase, pyruvate, and acetylcholine;

– diminished nerve-impulse transmission at synapses;– impaired DNA synthesis.– genetic predisposition may exist in some patients?

McEntee et al (1980) have demonstrated decreased levels of a metabolite of norepinephrine (3-methoxy-4-hydroxyphenolglycol or MHPG) in the CSF of some patients with Wernicke-Korsakoffsyndrome. Clonidine, an alpha-noradrenergic agonist, seemed to improve the memory disorder of their patients.

– However, these results have not been reproduced in any large prospective study. Patients with permanent Korsakoff psychosis are not treated routinely with clonidine.

Page 9: Wernicke Korsakoff Syndrome

Epidemiology

Long-standing alcohol use is the most common association with development ofWernicke-Korsakoff syndrome.– Prevalence data have come primarily from

necropsy studies, with rates of 1-3%, and have indicated that prevalence at autopsy exceeds recognition in life.

The mortality rate is 10-20%. Prognosis depends on the stage of disease at presentation and on prompt treatment.

Page 10: Wernicke Korsakoff Syndrome

Epi…

Race: No racial predilection is observed. Sex: The condition affects males slightly more frequently than females. Age: Age of onset is evenly distributed from 30-70 years.

Page 11: Wernicke Korsakoff Syndrome

Physical exam signs

Ocular abnormalities: can occur singly or in combination– Nystagmus, vertical and horizontal– Weakness or paralysis of lateral rectus muscles; Occurs bilaterally but can

be asymmetric and is accompanied by diplopia and internal strabismus– Weakness or paralysis of conjugate gaze– Nonreacting miotic pupils and complete loss of ocular movements (in

advanced cases)– Ptosis, small retinal hemorrhages, involvement of near-far focusing

mechanism, and optic neuropathy (occasionally)– Papilledema (very rare)

Ataxia is manifested as an abnormality of both stance and gait.Vestibular paresis also plays a role in ataxia in the early stages of disease.

– Mildest form evident on tandem walking only– Wide-based stance– Slow and uncertain short-stepped gait– In the most severe form, inability to walk without support– Abnormal results on caloric testing (indicates vestibular paresis)

Page 12: Wernicke Korsakoff Syndrome

Physical exam signs

A global confusional state is the most common early manifestation characterized by apathy, inattentiveness, and indifference to surroundings. General disorientation to time, place, and purpose. Stupor or coma can be observed in more severe cases but is rare as an initial presentation. If patients remain untreated, the condition will progress to death, as in the initial cases described by Wernicke.

– An amnestic state involves appropriate form and content with apparent loss of certain details.

– The Korsakoff state is characterized by both anterograde (ie, learning) and retrograde (ie, memory of past events) amnesia. These gaps in memory are what lead to the characteristic feature of confabulation.

Hypothermia presents secondary to damage in temperature regulating centers. Associated peripheral neuropathy is found in 80% of patients. Cardiovascular dysfunction may be observed. Overt signs of beriberi heart disease are rare; The following symptoms may be observed and generally improve with administration of thiamine:

– Postural hypotension, Tachycardia, Syncope

Page 13: Wernicke Korsakoff Syndrome

Ancillary testing

Wernicke encephalopathy remains a clinical diagnosis with no characteristic abnormalities in diagnostic studies of cerebrospinal fluid, brain imaging or EEGs.MRI of the brain with contrast:– May show acute lesions in medial thalamic, mamillary

bodies, and periaqueductal regions.

Page 14: Wernicke Korsakoff Syndrome

Causes to consider

Chronic alcoholismPersistent emesisHyperemesis gravidarumGastric malignancyIntestinal obstructionSystemic diseasesMalignancyDisseminated tuberculosisAcquired immunodeficiency syndromeUremia/Chronic hemodialysisStarvation/Refeeding after starvationAnorexia nervosaIatrogenicIntravenous hyperalimentation

Page 15: Wernicke Korsakoff Syndrome

Hyperemesis gravidarum

Quite a few case reports of Hyperemesis gravidarum associated with Wernicke-Korsakoff syndrome– At UNC: case report of a primipara with Hyperemesis gravidarum

admitted for MS changes at 14th week of pregnancy with opthalmoplegia, ataxia, hyporeflexia. Pt improved quickly with thiamine administration but ataxia persisted; spontaneous abortion occurred 2 weeks later.

• Obstetrics & Gynecology. 99(5 Pt 2): 875-7, 2002 May.

Page 16: Wernicke Korsakoff Syndrome

Treatment

Wernicke encephalopathy is a medical emergency.– Prompt recognition of the symptom complex and a high index of

suspicion are crucial.Intravenous thiamine (50-100 mg) is the treatment of choice.– Early treatment can rapidly reverse the ophthalmoplegia and

improve ataxia/dysequilibrium and early mental confusion, as well as prevent development of the amnestic state.

Mg, K supplements (often low in people with alcoholism).– Also, Mg is a necessary cofactor in thiamine-dependent

metabolism. Administration of intravenous glucose to patients who are severely malnourished can exhaust their supply of thiamine and precipitate Wernicke-Korsakoff syndrome.

Page 17: Wernicke Korsakoff Syndrome

Prognosis

Patients with Wernicke encephalopathy have a 10-20% mortality rate.In general, full recovery of ocular function occurs. Fine horizontalnystagmus can persist in as many as 60% of cases. Approximately 40% of patients have complete recovery from ataxia.Of patients surviving Wernicke encephalopathy, 80% have Korsakoffpsychosis.Of patients with Korsakoff psychosis, 25% do not recover and require long-term institutionalization. Only about 20% eventually recover completely during long-term follow-up care.Only 20% of patients recover completely from amnestic deficit.

Page 18: Wernicke Korsakoff Syndrome

Bibliography

Victor M, Martin JB: Nuitrional and metabolic diseases of the nervous system. In: Eugene Braunwald, ed. Harrison's Principles of Internal Medicine. 13th ed. New York, NY: McGraw Hill Text; 1994: 2328-2331. Reuler JB, Girard DE, Cooney TG: Current concepts.Wernicke's encephalopathy. N Engl J Med 1985 Apr 18; 312(16): 1035-9Obstetrics & Gynecology. 99(5 Pt 2): 875-7, 2002 May. http://www.uic.edu/depts/mcne/homepage/neurofounders.htmlhttp://www.emedicine.com/med/topic2405.htmhttp://www.emedicine.com/emerg/topic642.htm

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HAPPY EASTER!


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